{"title":"Claudin18.2 expression in gallbladder cancer correlates with immune activation and a favourable prognosis.","authors":"Shu-Juan Ni, Xin Wang, Lin Yuan, Hui Dong, Hui Sun, Cong Tan, Xu Cai, Wenhua Jiang, Weiqi Sheng, Midie Xu, Dan Huang","doi":"10.1136/jcp-2024-209914","DOIUrl":"https://doi.org/10.1136/jcp-2024-209914","url":null,"abstract":"<p><strong>Aims: </strong>Gallbladder carcinoma (GBC) is frequently diagnosed and treated in advanced stages and has a poor prognosis. Recent studies have identified claudin18.2 (CLDN18.2) as a promising target in digestive system cancer. In this study, we aimed to determine the expression of CLDN18.2 and its correlation with clinicopathological characteristics in patients with GBC.</p><p><strong>Methods: </strong>The expression of CLDN18.2 of 228 patients with GBC was studied via immunohistochemistry. Immunostained samples were evaluated according to the H-score. The samples were divided into low/negative (H-score=0-49) and high/positive (H-score=50-300) expression groups. The correlations between CLDN18.2 and various clinicopathological characteristics, including survival, were assessed. Multiplex immunofluorescence and image acquisition were used to analyse the relationship between CLDN18.2 expression and the immune microenvironment.</p><p><strong>Results: </strong>The overall positive CLDN18.2 staining rate was 39.91% (91/228); 137 (60.08%) were given 0 points, 30 (13.15%) were given 1 point, 28 (12.28%) were given 2 points and 33 (14.47%) were given 3 points. Low CLDN18.2 expression was correlated with adverse prognostic factors, including poor differentiation, deep infiltration depth, lymph node metastasis and distant metastasis. High CLDN18.2 expression was associated with better survival. Furthermore, the distribution of immune cell subsets significantly differed between the high and low CLDN18.2 expression groups.</p><p><strong>Conclusions: </strong>The correlations between the expression of CLDN18.2 and clinicopathological characteristics and prognosis suggest that early-stage patients could benefit more from future anti-CLDN18.2 treatment and that CLDN18.2 may function as a pivotal regulatory molecule in patients with GBC. The underlying mechanism may be related to immune activation caused by high CLDN18.2 expression.</p>","PeriodicalId":15391,"journal":{"name":"Journal of Clinical Pathology","volume":" ","pages":""},"PeriodicalIF":2.5,"publicationDate":"2025-02-13","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"143414318","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":4,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
{"title":"Fifth edition WHO classification: myeloid neoplasms.","authors":"Xueyan Chen, Nikhil Patkar, Prashant Tembhare, Subramanian Papagudi, Cecelia Yeung, Rashmi Kanagal Shamanna, Sumeet Gujral, Brent Wood, Kikkeri N Naresh","doi":"10.1136/jcp-2024-210022","DOIUrl":"https://doi.org/10.1136/jcp-2024-210022","url":null,"abstract":"<p><p>The fifth edition of the WHO classification of haematolymphoid tumours (WHO-HEM5) introduces significant advancements in the understanding and diagnosis of myeloid neoplasms, emphasising molecular and genetic insights. This review highlights key updates from the revised fourth edition (WHO-HEM4R), particularly the integration of genetic criteria for disease classification. Many entities are now defined by specific genetic abnormalities, enhancing diagnostic precision and prognostic assessment. Notably, the elimination of the 20% blast threshold for acute myeloid leukaemia (AML) with specific defining genetic alterations reflects a shift towards genomic-driven diagnostics. Additional updates include the refined subclassification of myelodysplastic neoplasms (MDS) and MDS/myeloproliferative neoplasms, as well as the recognition of novel entities such as clonal haematopoiesis and MDS with biallelic TP53 inactivation, further expanding the spectrum of myeloid neoplasms. WHO-HEM5 illustrates the diagnostic utility of morphology, flow cytometry, immunohistochemistry and next-generation sequencing in resource-rich settings. However, its implementation in low-income and middle-income countries (LMICs) remains challenging due to limited access to advanced diagnostic tools. This review explores strategies to optimise diagnosis in resource-constrained environments, where morphology and immunophenotyping remain fundamental. By integrating molecular diagnostics with traditional methods, WHO-HEM5 aims to refine classification and facilitate risk stratification in the era of personalised medicine, providing haematopathologists and clinicians with an essential framework to navigate the complexities of myeloid neoplasms. The emphasis on advancing haematopathology practices worldwide, including in LMICs, demonstrates the ongoing commitment to improving global outcomes in haematological malignancies.</p>","PeriodicalId":15391,"journal":{"name":"Journal of Clinical Pathology","volume":" ","pages":""},"PeriodicalIF":2.5,"publicationDate":"2025-02-13","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"143414269","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":4,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
Carlos E De Andrea, Alvaro Lopez-Janeiro, Pancras C W Hogendoorn
{"title":"New evidence for fibrocartilaginous dysplasia representing a variant of fibrous dysplasia.","authors":"Carlos E De Andrea, Alvaro Lopez-Janeiro, Pancras C W Hogendoorn","doi":"10.1136/jcp-2024-210007","DOIUrl":"https://doi.org/10.1136/jcp-2024-210007","url":null,"abstract":"<p><p>Several types of tumours and tumour-like lesions are recognised. Their classification is based mainly on the cell or tissue differentiation pathway found within the lesion. Not all tumour cells in a bone tumour differentiate towards a single cell type. For instance, cartilage-producing cells and cartilaginous matrix are often found within osteosarcoma, and on rare occasions, cartilage can be found in fibrous dysplasia. Here, we discuss the presence of cartilaginous differentiation with fibrous dysplasia, its differential diagnosis and the use of molecular techniques to show that cartilaginous differentiation is an integral part of the lesion in that case, also known as fibrocartilaginous variant of fibrous dysplasia.</p>","PeriodicalId":15391,"journal":{"name":"Journal of Clinical Pathology","volume":" ","pages":""},"PeriodicalIF":2.5,"publicationDate":"2025-02-06","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"143364935","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":4,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
{"title":"Spindle cell carcinoma of the breast resembling pseudoangiomatous stromal hyperplasia.","authors":"Andrew H S Lee, Anne-Marie O'Shea, Ian O Ellis","doi":"10.1136/jcp-2024-209979","DOIUrl":"https://doi.org/10.1136/jcp-2024-209979","url":null,"abstract":"<p><p>Pseudoangiomatous stromal hyperplasia (PASH) is commonly present in gynaecomastia, can be seen in some mammary fibroepithelial lesions and in fibrocystic change, and rarely forms a mass. Spindle cell carcinoma of the breast can have a wide range of appearances. This case series describes five spindle cell carcinomas of the breast resembling PASH, a pattern that does not appear to have been reported before. All had bland nuclei like those in fibromatosis-like spindle cell carcinoma. All cases had more close-packed areas, but this was only a very minor component in the index case. Two had associated ductal carcinoma in situ. All were cytokeratin positive with immunohistochemistry. The similarity of the carcinomas in this series to PASH is a diagnostic pitfall particularly in core biopsies as more cellular areas may be only focal.</p>","PeriodicalId":15391,"journal":{"name":"Journal of Clinical Pathology","volume":" ","pages":""},"PeriodicalIF":2.5,"publicationDate":"2025-02-04","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"143189217","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":4,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
Eva A M Hesius, Wendy B C Stevens, James P Stewart, Leonie I Kroeze, Ellen van der Spek, Djamila Issa, Peet Nooijen, Jeroen Luijks, David Gonzalez, Patricia J T A Groenen, Nicole M A Blijlevens, Annemiek B van Spriel, Michiel van den Brand
{"title":"Mutational profile dynamics in follicular lymphoma and large cell transformation.","authors":"Eva A M Hesius, Wendy B C Stevens, James P Stewart, Leonie I Kroeze, Ellen van der Spek, Djamila Issa, Peet Nooijen, Jeroen Luijks, David Gonzalez, Patricia J T A Groenen, Nicole M A Blijlevens, Annemiek B van Spriel, Michiel van den Brand","doi":"10.1136/jcp-2024-209880","DOIUrl":"https://doi.org/10.1136/jcp-2024-209880","url":null,"abstract":"<p><strong>Aims: </strong>Follicular lymphoma (FL) is characterised by significant heterogeneity in both the clinical trajectories and the molecular profiles. This study aimed to investigate clonal dynamics in FL by analysing mutation profiles at various time points during the disease course including at histological transformation (HT), to gain insight into the mutational changes over time.</p><p><strong>Methods: </strong>We retrospectively analysed 76 biopsies from 25 patients, including 13 cases with three or more FL biopsies and 12 cases with subsequent HT. Hybrid capture-based Next-Generation Sequencing (NGS) with the EuroClonality-NGS DNA capture (EuroClonality-NDC) assay was used to examine clonal rearrangements and mutations.</p><p><strong>Results: </strong>A total of 204 (potentially) pathogenic mutations were identified. Only 40% of mutations remained stably present during a median follow-up period of 139 months (range 9-198). <i>KMT2D</i> and <i>CREBBP</i> were the most frequently mutated genes at diagnosis, exhibiting relative stability in follow-up biopsies. Conversely, <i>EZH2</i> displayed a dynamic pattern of mutations gained and lost during the disease course. At HT, pathogenic mutations affecting <i>B2M</i>, <i>MYC</i> and <i>TP53</i> emerged. Changes in mutational burden were observed in both FL-sequential and diagnosis-transformation cohorts, with more pronounced changes in the latter.</p><p><strong>Conclusions: </strong>This real-world study provides insights into the complex molecular pathogenesis of FL and HT. As targeted therapies emerge as treatment modalities, mutational profiles could influence treatment decisions in the future. Therefore, recognising the significant changes occurring in the mutational landscape of FL throughout the disease course is crucial.</p>","PeriodicalId":15391,"journal":{"name":"Journal of Clinical Pathology","volume":" ","pages":""},"PeriodicalIF":2.5,"publicationDate":"2025-01-31","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"143074708","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":4,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
Isabella Caroline Chiara Niesner, Kevin Jon Balbi, Benjamin Poskitt, Carolina Gemma, Josep Linares, David Allen, Oliver Shutkever, Colin R Lindsay, Philip Bennett, David Allan Moore
{"title":"Prevalence and breakdown of <i>KRAS</i> driver mutations in a large UK non-small cell lung cancer cohort.","authors":"Isabella Caroline Chiara Niesner, Kevin Jon Balbi, Benjamin Poskitt, Carolina Gemma, Josep Linares, David Allen, Oliver Shutkever, Colin R Lindsay, Philip Bennett, David Allan Moore","doi":"10.1136/jcp-2024-209972","DOIUrl":"https://doi.org/10.1136/jcp-2024-209972","url":null,"abstract":"<p><p>Kirsten rat sarcoma viral oncogene (<i>KRAS</i>) is a frequently mutated oncogene in lung cancer, now amenable to targeted therapy with allele-specific G12C inhibitors. Non-small cell lung cancer (NSCLC) driver mutations show geographical variability and therefore the <i>KRAS</i> mutation breakdown, co-occurring oncogenic mutation rate and associated PD-L1 expression were studied in a large UK cohort. We interrogated archival clinical next-generation sequencing (NGS) data over 5 years. 3283 NSCLC samples were included, referred from 38 centres over 4 years. Somatic mutation hotspots in cancer-associated genes were analysed using ampliseq/ion-torrent based NGS assays. In a subset of the cohort, PD-L1 scores were also collated. 1118 <i>KRAS</i> variants were detected. Class I mutations occurred most frequently (86.94%), with <i>KRAS</i> G12C, G12V and G12D being most prevalent. Class II (7.96%), III (4.65%) and IV (0.45%) mutations were also detected and mutations in <i>PIK3CA</i> and <i>BRAF</i> were the most frequently observed co-mutations. No significant difference in PD-L1 expression was found between <i>KRAS</i> wild-type and mutant tumours.</p>","PeriodicalId":15391,"journal":{"name":"Journal of Clinical Pathology","volume":" ","pages":""},"PeriodicalIF":2.5,"publicationDate":"2025-01-31","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"143074740","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":4,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
{"title":"The prevalence of PD-L1 expression in patients with advanced oesophageal cancer: the EXCEED observational study.","authors":"Liyan Xue, Jiaqi Wang, Dong Kuang, Jingping Yun, Yuan Li, Lili Jiang, Daoyuan Wu, Pei Duan, Shixun Lu, Yan Jin, Du He, Jing Qian, Wenmin Tang, Yan Wang, Jielin Li, Jianming Ying","doi":"10.1136/jcp-2024-209721","DOIUrl":"https://doi.org/10.1136/jcp-2024-209721","url":null,"abstract":"<p><strong>Aims: </strong>There are limited data on programmed death ligand 1 (PD-L1) expression in oesophageal cancer (OC) from multicentre studies conducted across China. We aimed to determine the prevalence of high PD-L1 expression in patients with advanced OC.</p><p><strong>Methods: </strong>The EXCEED study was a multicentre, retrospective analysis of data from six tertiary hospitals that evaluated PD-L1 expression in adults with advanced OC or advanced head and neck squamous cell carcinoma. PD-L1 expression was evaluated at each site according to a standardised protocol. The primary outcome was the prevalence of high PD-L1 expression (Combined Positive Score (CPS) ≥10) in surgical or tumour biopsy samples. Low PD-L1 expression was defined as CPS <10. Patient demographic and baseline factors associated with high PD-L1 expression were also investigated. This report presents the results for the OC cohort only.</p><p><strong>Results: </strong>Overall, 482 patients were included, the majority were male (87.6%) and the mean age at diagnosis was 63.3 years; 207 had high PD-L1 expression (42.9%; 95% CI 38.5, 47.5) and 275 had low expression (57.1%; 95% CI 52.5, 61.5). There were significant differences in high PD-L1 expression prevalence between subgroups by sex (p=0.044), number of distant metastases (p=0.020), and if chemotherapy (p=0.004) was received prior to the collection of biological samples (ie, biopsy or surgery).</p><p><strong>Conclusions: </strong>These real-world data provide a robust estimate of the prevalence of high PD-L1 expression in patients with advanced OC and identify clinicopathological and treatment features related to PD-L1 expression that can inform treatment selection.</p>","PeriodicalId":15391,"journal":{"name":"Journal of Clinical Pathology","volume":" ","pages":""},"PeriodicalIF":2.5,"publicationDate":"2025-01-28","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"143059326","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":4,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
Antonio Santisteban-Espejo, Cristian Benavides-De la Fuente, Alipio Mangas-Rojas, Pedro Montero-Pavon, Irene Bernal-Florindo, Eduardo Aldaco-Puntas, Isabel Prieto-Conde, Jose Perez-Requena, Lidia Atienza-Cuevas, Maria Del Carmen Fernández-Valle, Sebastian Garzón-López, Marcial Garcia-Rojo
{"title":"Computational pathology identifies a low B-cell content in the tumour microenvironment as a predictor of adverse outcome in patients with classic Hodgkin lymphoma treated with ABVD.","authors":"Antonio Santisteban-Espejo, Cristian Benavides-De la Fuente, Alipio Mangas-Rojas, Pedro Montero-Pavon, Irene Bernal-Florindo, Eduardo Aldaco-Puntas, Isabel Prieto-Conde, Jose Perez-Requena, Lidia Atienza-Cuevas, Maria Del Carmen Fernández-Valle, Sebastian Garzón-López, Marcial Garcia-Rojo","doi":"10.1136/jcp-2024-209848","DOIUrl":"https://doi.org/10.1136/jcp-2024-209848","url":null,"abstract":"<p><strong>Aims: </strong>The prognostic impact of B lymphocytes surrounding Hodgkin and Reed Sternberg (HRS) cells in classic Hodgkin lymphoma (cHL) and pathogenic variants in genes associated with apoptosis regulation remains undefined.</p><p><strong>Methods: </strong>We have quantified the proportion of B lymphocytes in tumour microenvironment (TME) in 220 diagnostic slides from 110 cHL patients applying computational pathology (CP) and sequenced cases using a targeted panel including 47 genes recurrently mutated in mature B-cell neoplasms. Kaplan-Meier estimators and multivariate Cox regression on overall survival (OS) and progression-free survival (PFS) were assessed following the Transparent Reporting of a multivariable prediction model for Individual Prognosis Or Diagnosis guidelines.</p><p><strong>Results: </strong>The mean percentage of B lymphocytes was 45.1 (SD: 24.8). Genes recurrently affected by nonsynonymous somatic mutations in 25% or more of patients included EP300, NOTCH and ABL1. A lower number of mutations were discovered in Epstein-Barr virus-positive cHL (21.1% vs 78.8%) reinforcing the notion that viral infection could functionally replace the need for genomic aberrations. Classic Hodgkin lymphoma (cHL) patients that jointly presented a reduction in the number of B lymphocytes in TME (<8%) and the absence of mutations in apoptosis-associated genes (ABL1, BIRC3, CASP8 and FAS) presented a lower OS (mean OS: 31.5 months, 95% CI: 0 to 69.7 months) in comparison with patients without this event (mean OS: 84.7 months, 95% CI: 61.9 to 107.5 months) (p=0.01). This high-risk cHL subgroup also presented a significantly lower PFS (mean PFS: 8.5 months, 95% CI: 7.5 to 9.5 months) in comparison with B-cell-enriched or apoptosis-mutated cHL (mean PFS: 55.2 months; 95% CI: 42.4 to 68 months) (p<0.001).</p><p><strong>Conclusions: </strong>This study expands previous data on the value of CP in cHL, and specifically, the distribution of B cells, identifying patients with an increased risk of treatment failure and progression. Furthermore, immune escape by apoptosis dysregulation during clonal selection occurring in germinal centres constitutes a landmark of cHL. These results could be the basis for further development of targeted therapies directed against apoptosis modulators in cHL.</p>","PeriodicalId":15391,"journal":{"name":"Journal of Clinical Pathology","volume":" ","pages":""},"PeriodicalIF":2.5,"publicationDate":"2025-01-20","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"143006209","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":4,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
Yu Huang, Jinnan Chen, Yixian Guo, Zhaohui Ding, Xiao Liang, Wei Zhang, Hanbing Xue, Yunjia Zhao, Xiaobo Li, Hong Lu
{"title":"Staging of operative link on gastritis assessment and operative link on gastric intestinal metaplasia systems for risk assessment of early gastric cancer: a case-control study.","authors":"Yu Huang, Jinnan Chen, Yixian Guo, Zhaohui Ding, Xiao Liang, Wei Zhang, Hanbing Xue, Yunjia Zhao, Xiaobo Li, Hong Lu","doi":"10.1136/jcp-2023-209209","DOIUrl":"10.1136/jcp-2023-209209","url":null,"abstract":"<p><strong>Aims: </strong>Operative link on gastritis assessment (OLGA) and operative link on gastric intestinal metaplasia assessment (OLGIM) systems are histological staging systems of gastritis for gastric cancer (GC) risk estimation. Intermediate OLGA/OLGIM stages are of concern in a region with high incidence of GC. This study aimed to validate OLGA and OLGIM staging systems for early GC (EGC) in Chinese population.</p><p><strong>Methods: </strong>This single-centre, case-control study included 196 patients with EGC and 196 age-matched and sex-matched health screening control subjects. OLGA and OLGIM systems, and other clinical parameters were evaluated using logistic regression analysis.</p><p><strong>Results: </strong>OLGA and OLGIM stages II/III/IV were more prevalent in patients with EGC than in the control subjects. Multivariable analysis revealed family history of GC, previous <i>Helicobacter pylori</i> (<i>H. pylori</i>) infection, OLGA stages II and III-IV, OLGIM stages II and III-IV as independent risk factors for EGC (ORs, 4.04, 1.87, 2.52, 6.79, 4.11 and 10.78, respectively). Area under the receiver operating characteristic curve on EGC risk estimation was improved for OLGIM compared with OLGA (0.78 vs 0.71, p<0.001). Autoantibody seropositivity of gastric mucosa was not associated with EGC risk stratified by <i>H. pylori</i> status.</p><p><strong>Conclusions: </strong>Surveillance of intermediate-risk patients (OLGA/OLGIM II) should be emphasised in our region. The OLGIM may be preferred over the OLGA for EGC risk estimation.</p>","PeriodicalId":15391,"journal":{"name":"Journal of Clinical Pathology","volume":" ","pages":"117-122"},"PeriodicalIF":2.5,"publicationDate":"2025-01-17","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"138291095","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":4,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
Miriam Duci, Luisa Santoro, Angelo Paolo Dei Tos, Greta Loss, Claudia Mescoli, Piergiorgio Gamba, Francesco Fascetti Leon
{"title":"Postoperative Hirschsprung's associated enterocolitis (HAEC): transition zone as putative histopathological predictive factor.","authors":"Miriam Duci, Luisa Santoro, Angelo Paolo Dei Tos, Greta Loss, Claudia Mescoli, Piergiorgio Gamba, Francesco Fascetti Leon","doi":"10.1136/jcp-2023-209129","DOIUrl":"10.1136/jcp-2023-209129","url":null,"abstract":"<p><strong>Aims: </strong>Hirschsprung's-associated enterocolitis (HAEC) is the most severe complication of Hirschsprung disease (HD), and its pathogenesis is still unknown. Length of transition zone (TZ) interposed between aganglionic and normal bowel has been poorly explored as predictor for postoperative HAEC (post-HAEC). This study aimed to identify potential predictive factors for post-HAEC, with a particular focus on histopathological findings.</p><p><strong>Methods: </strong>Data from Hirschsprung patients treated in a single Italian centre between 2010 and 2022 with a follow-up >6 months were collected. Thorough histopathological examination of the resected bowel was conducted, focusing on length of TZ and aganglionic bowel.The degree of inflammatory changes in ganglionic resected bowel was further obtained. Ultra-long HD, total colonic aganglionosis and ultra-short HD were excluded. Bivariate and multivariate regression analysis were performed.</p><p><strong>Results: </strong>Thirty-one patients were included; 5 experienced preoperative HAEC (pre-HAEC) and later post-HAEC (16.1%), further 10 patients developed post-HAEC (total post-HAEC 48.38%). Pre-HAEC-history and a TZ<2.25 cm correlated with an early development of post-HAEC. Multivariate analysis identified a TZ<2.25 cm as an independent post-HAEC predictive factor (p=0.0096). Inflammation within the ganglionic zone and a TZ<2.25 cm correlated with higher risk of post-HAEC (p=0.0074, 0.001, respectively). Severe post-HAEC more frequently occurred in patients with pre-HAEC (p=0.011), histological inflammation (p=0.0009) and short TZ (p=0.0015).</p><p><strong>Conclusions: </strong>This study suggests that TZ<2.25 cm predicts the risk of post-HAEC. Preoperative clinical and histopathology inflammation may predispose to worst post-HAEC. Readily available histopathological findings might help identifying patients at higher risk for HAEC and implementing prevention strategies.</p>","PeriodicalId":15391,"journal":{"name":"Journal of Clinical Pathology","volume":" ","pages":"111-116"},"PeriodicalIF":2.5,"publicationDate":"2025-01-17","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"138487648","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":4,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}